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Home  >  Medical Research Archives  >  Issue 149  > Neuroendocrine, Autonomic and Metabolic Challenges in Hypermobile Ehlers-Danlos Syndrome: A Case Study on Hypoglycemia in a patient with Craniocervical Instability.
Published in the Medical Research Archives
Jun 2024 Issue

Neuroendocrine, Autonomic and Metabolic Challenges in Hypermobile Ehlers-Danlos Syndrome: A Case Study on Hypoglycemia in a patient with Craniocervical Instability.

Published on Jun 15, 2024

DOI 

Abstract

 

This report presents a compelling case of hypoglycemia in a 20-year-old female with craniocervical instability receiving chronic total parenteral nutrition (TPN). Noteworthy is her intricate history, including Chiari malformation, Hypermobile Ehlers-Danlos syndrome, and tethered cord, complicating the clinical picture. 

Following a complex spinal surgery, the patient received postoperative dexmedetomidine for pain relief. Subsequent hypoglycemic episodes prompted meticulous investigations and endocrinology consultations. Discrepancies in TPN infusion rates, nephrology perspective on glycogen depletion due to chronic TPN, and the patient's unique medical history added layers of complexity to the clinical landscape. 

Our exploration delves into the multifactorial nature of hypoglycemia in this patient. Chronic TPN alters glucose dynamics, impacting glycogen stores, while dexmedetomidine, known for α2-adrenoceptor activation induced sympatholysis, may contribute to hypoglycemia. Dysfunction of hypothalamic-pituitary axis in patients with craniocervical instability necessitated enhanced scrutiny for the detection of potential central etiologies of hypoglycemia. 

Our findings underscore the importance of a multidisciplinary approach, integrating pharmacological insights, nutritional considerations, and the patient's unique medical history, to provide a comprehensive understanding of adverse events in complex clinical scenarios. The dynamic nature of glycemic control in this context warrants careful consideration in clinical decision-making.

Glossary of Items: Total parenteral nutrition (TPN), dextrose 50% in water (D50), dextrose 20% in water (D20), dextrose 10% in water (D10), Adrenocorticotropic Hormone (ACTH), Cytochrome P450 (CYP).

Author info

Navdeep Nayyar, Paolo Bolognese, Jaclyn Amaru, June Guo, Lance Cho, Sophie Bloom, John Biggins, Ilene Ruhoy

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